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Fears grow over 'mad elk disease'

s are looking. For example, Pierluigi Gambetti of the National Prion Disease Pathology Surveillance Center in Cleveland, Ohio, has found that between 1997 and 2002 about 60 per cent of the 921 suspect cases were confirmed as being a prion disease. But these cases amount to fewer than a third of the number that would be expected for the same period based on the rates of sporadic and familial CJD elsewhere, he will tell the Honolulu meeting this week.

That shows how ineffective the surveillance is, says Norman Foster, a neurologist at the University of Michigan in Ann Arbor. So if CWD-contaminated venison does trigger a new form of CJD in people, there could be dozens of extra cases of CJD without them being detected.

Part of the problem is that CJD resembles common diseases such as dementia in the elderly. Doctors have no set guidelines on what to look for and only autopsies can confirm a diagnosis. But few are carried out and CJD is not a reportable disease in the US.

Autopsies can also distinguish between sporadic CJD and the variant form triggered by mad cow disease. But since no one knows what the brains of people with "CWD-CJD" would look like- if the disease does indeed exist- it is hard for pathologists to be sure that a particular CJD case is not related to CWD.

To compound matters further, different federal agencies are responsible for surveillance in humans and animals. Foster and others are pushing for a surveillance programme similar to Britain's mad cow disease and CJD surveillance programmes.


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Contact: Claire Bowles
claire.bowles@rbi.co.uk
207-331-2751
New Scientist
2-Apr-2003


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